RESUMEN
The aims of the study were to assess efficacy and safety of TNF-alpha antagonists (anti-TNF) in a cohort of patients with juvenile idiopathic arthritis (JIA) who began treatment under 4 years old and to assess relapse rate after methotrexate and/or anti-TNF withdrawal. We made a retrospective charts review of our non-systemic JIA patients treated with anti-TNF under 4 years of age between January 2006 and April 2013. Demographics, epidemiologic, clinical, laboratory data and rate of relapse after treatment withdrawal due to clinical remission were collected. Efficacy and safety end points included side effects (SE) and time to achieve clinical remission. We included 27 patients, 23 received etanercept and 4 adalimumab with a median age of 3.01 (range 0.88-3.97) years at anti-TNF beginning and 1.94 (range 0.18-5.44) and 2.39 (range 0.18-7.24) years of treatment and follow-up, respectively. All patients had previously received disease-modifying antirheumatic drugs at optimal dose. Nineteen patients reached clinical remission on treatment in a median time of 9.1 (range 6.23-21.17) months. Four of those relapsed during treatment. Six developed mild SE, mostly mild infections. No serious SE were described. Eleven patients who reached clinical remission relapsed after treatment withdrawal. None achieved clinical remission off treatment. Most patients reached clinical remission on anti-TNF. In our cohort of patients, etanercept and adalimumab were safe, with mostly mild infections and no serious SE. We observed a high relapse rate during treatment withdrawal.
Asunto(s)
Anticuerpos Monoclonales Humanizados/uso terapéutico , Antirreumáticos/uso terapéutico , Artritis Juvenil/tratamiento farmacológico , Inmunoglobulina G/uso terapéutico , Receptores del Factor de Necrosis Tumoral/uso terapéutico , Factor de Necrosis Tumoral alfa/antagonistas & inhibidores , Adalimumab , Factores de Edad , Preescolar , Quimioterapia Combinada , Etanercept , Femenino , Humanos , Lactante , Masculino , Metotrexato/uso terapéutico , Recurrencia , Inducción de Remisión , Estudios Retrospectivos , Resultado del Tratamiento , Privación de TratamientoRESUMEN
TNF-alpha-blocking agents (anti-TNF) used in juvenile idiopathic arthritis (JIA) are well established; however, time to withdraw is unclear. Neither prolonged nor tapering treatment seems to influence risk of relapse. Our aim was to assess relapse percentage after anti-TNF withdrawal of our non-systemic JIA patients after reaching clinical remission. A retrospective review of our non-systemic JIA patients in whom anti-TNF had been withdrawn due to inactive disease was achieved, between December 2000 and November 2011. We analyzed percentages of relapse according to JIA categories and antinuclear antibodies (ANA) positivity. n = 18 patients were included. Eighty-two percentage of patients relapsed after treatment withdrawal, and mean time to relapse was 3.04 months (SD 2.03). The percentage of relapse after anti-TNF discontinuation in the main JIA category was 88 % of negative rheumatoid factor polyarticular JIA and 80 % of persistent oligoarticular JIA. We did not find significant statistical differences according to ANA positivity (9 of 14 were ANA positive), and mean time to relapse (days) was 85.0 (SD 69.4) for ANA-positive versus 102.4 (SD 47.7) for ANA-negative patients (p = NS). Relapse percentage following anti-TNF discontinuation was high (82 %) and occurred within the first 3 months after it. No relationship regarding JIA subtype and ANA positivity was found.
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Antirreumáticos/administración & dosificación , Artritis Juvenil/tratamiento farmacológico , Artritis/tratamiento farmacológico , Factor de Necrosis Tumoral alfa/antagonistas & inhibidores , Anticuerpos Antinucleares/sangre , Artritis/sangre , Artritis/diagnóstico , Artritis/inmunología , Artritis Juvenil/sangre , Artritis Juvenil/diagnóstico , Artritis Juvenil/inmunología , Biomarcadores/sangre , Esquema de Medicación , Humanos , Recurrencia , Inducción de Remisión , Estudios Retrospectivos , Factor Reumatoide/sangre , Factores de Riesgo , Factores de Tiempo , Resultado del TratamientoRESUMEN
Key Clinical Message: The presence of more than one genetic/genomic disorder is not uncommon. It is therefore essential to continuously consider new signs and symptoms over time. Administration of gene therapy could be extremely difficult in particular situations. Abstract: A 9-month-old boy presented to our department for evaluation of developmental delay. We found that he was affected by intermediate junctional epidermolysis bullosa (COL17A1, c.3766 + 1G > A, homozygous), Angelman syndrome (5,5 Mb deletion of 15q11.2-q13.1), and autosomal recessive deafness type 57 (PDZD7, c.883C > T, homozygous).
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INTRODUCTION: The number of patients with complex chronic conditions (CCC) has increased in the last 20 years or so. There is limited data as regards the prevalence of CCC in the paediatric population and its impact on hospital admissions. The main objectives of this study are to determine the proportion of CCC in the paediatric hospital population and compare them with other groups of patients admitted (acute and chronic). PATIENTS AND METHODS: A descriptive, retrospective study was carried out in a tertiary maternity-paediatric hospital (from December 2016 to November 2017). All patients admitted into the Paediatric Department were recruited with a fortnightly frequency. A series of demographic, clinical, and pregnancy data were collected. In order to identify the level of complexity of the patients, the Clinical Risk Group (CRG) was used, with 3 groups being created: acute, chronic, and CCC. Statistics analysis was performed using SPSS v24. RESULTS: A total of 1433 patients were included. The proportion of CCC on the Paediatric Ward was 14.4%. The CCC were older patients, mainly admitted due to decompensation or progression of their underlying disease, had a longer admission time, and required support in the Paediatric Intensive Care Unit (PICU) more often than that of the other sub-groups. Just under half (44.7%) of the CCC were carriers of a technological device. Of the total of long stays (>1 month), 71.3% had CCC. CONCLUSIONS: Patients with CCC require long hospital stays, a greater need of intensive care, and use of technology. New approaches to treatment and follow-up need to be established. They should be cost-effective, and at the same time decrease the impact of the disease on the children and their family.
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Hospitalización , Unidades de Cuidado Intensivo Pediátrico , Niño , Enfermedad Crónica , Femenino , Humanos , Tiempo de Internación , Embarazo , Estudios RetrospectivosRESUMEN
Infection by rhinovirus (RV) and enterovirus (EV) in children ranges from asymptomatic infection to severe lower respiratory tract infection (LRTI). This cohort study evaluates the clinical impact of RV/EV species, alone or in codetection with other viruses, in young children with severe LRTI. Seventy-one patients aged less than 5 years and admitted to the Paediatric Intensive Care Unit (PICU) of a reference children's hospital with RV or EV (RV/EV) LRTI were prospectively included from 1/2018 to 3/2020. A commercial PCR assay for multiple respiratory pathogens was performed in respiratory specimens. In 22/71, RV/EV + respiratory syncytial virus (RSV) was found, and 18/71 had RV/EV + multiple viral detections. Patients with single RV/EV detection required invasive mechanical ventilation (IMV) as frequently as those with RSV codetection, whereas none of those with multiple viral codetections required IMV. Species were determined in 60 samples, 58 being RV. No EV-A, EV-C, or EV-D68 were detected. RV-B and EV-B were only found in patients with other respiratory virus codetections. There were not any associations between RV/EV species and severity outcomes. To conclude, RV/EV detection alone was observed in young children with severe disease, while multiple viral codetections may result in reduced clinical severity. Differences in pathogenicity between RV and EV species could not be drawn.
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Coinfección/virología , Cuidados Críticos , Infecciones por Enterovirus/virología , Enterovirus , Infecciones del Sistema Respiratorio/virología , Preescolar , Coinfección/epidemiología , Enterovirus Humano D , Infecciones por Enterovirus/epidemiología , Femenino , Hospitalización , Humanos , Lactante , Recién Nacido , Masculino , Virus Sincitial Respiratorio Humano , Infecciones del Sistema Respiratorio/diagnóstico , Infecciones del Sistema Respiratorio/epidemiologíaRESUMEN
Multisystem inflammatory syndrome associated with the SARS-CoV-2 pandemic has recently been described in children (MIS-C), partially overlapping with Kawasaki disease (KD). We hypothesized that (a) MIS-C and prepandemic KD cytokine profiles may be unique and justify the clinical differences observed, and (b) SARS-CoV-2-specific immune complexes (ICs) may explain the immunopathology of MIS-C. Seventy-four children were included: 14 with MIS-C, 9 patients positive for SARS-CoV-2 by PCR without MIS-C (COVID), 14 with prepandemic KD, and 37 healthy controls (HCs). Thirty-four circulating cytokines were quantified in pretreatment serum or plasma samples and the presence of circulating SARS-CoV-2 ICs was evaluated in MIS-C patients. Compared with HCs, the MIS-C and KD groups showed most cytokines to be significantly elevated, with IFN-γ-induced response markers (including IFN-γ, IL-18, and IP-10) and inflammatory monocyte activation markers (including MCP-1, IL-1α, and IL-1RA) being the main triggers of inflammation. In linear discriminant analysis, MIS-C and KD profiles overlapped; however, a subgroup of MIS-C patients (MIS-Cplus) differentiated from the remaining MIS-C patients in IFN-γ, IL-18, GM-CSF, RANTES, IP-10, IL-1α, and SDF-1 and incipient signs of macrophage activation syndrome. Circulating SARS-CoV-2 ICs were not detected in MIS-C patients. Our findings suggest a major role for IFN-γ in the pathogenesis of MIS-C, which may be relevant for therapeutic management.
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COVID-19/etiología , Citocinas/sangre , Síndrome Mucocutáneo Linfonodular/etiología , Síndrome de Respuesta Inflamatoria Sistémica/etiología , Adolescente , Anticuerpos Antivirales/sangre , Complejo Antígeno-Anticuerpo/sangre , Antígenos Virales/sangre , COVID-19/inmunología , COVID-19/virología , Estudios de Casos y Controles , Niño , Preescolar , Estudios de Cohortes , Estudios Transversales , Femenino , Humanos , Lactante , Interferón gamma/sangre , Masculino , Modelos Inmunológicos , Síndrome Mucocutáneo Linfonodular/inmunología , Pandemias , SARS-CoV-2/genética , SARS-CoV-2/inmunología , Síndrome de Respuesta Inflamatoria Sistémica/inmunología , Síndrome de Respuesta Inflamatoria Sistémica/virologíaRESUMEN
INTRODUCTION: The number of patients with complex chronic conditions (CCC) has increased in the last 20 years or so. There is limited data as regards the prevalence of CCC in the paediatric population and its impact on hospital admissions. The main objectives of this study are to determine the proportion of CCC in the paediatric hospital population and compare them with other groups of patients admitted (acute and chronic). PATIENTS AND METHODS: A descriptive, retrospective study was carried out in a tertiary maternity-paediatric hospital (from December 2016 to November 2017). All patients admitted into the Paediatric Department were recruited with a fortnightly frequency. A series of demographic, clinical, and pregnancy data were collected. In order to identify the level of complexity of the patients, the Clinical Risk Group (CRG) was used, with 3groups being created: acute, chronic, and CCC. Statistics analysis was performed using SPSS v24. RESULTS: A total of 1,433 patients were included. The proportion of CCC on the Paediatric Ward was 14.4%. The CCC were older patients, mainly admitted due to decompensation or progression of their underlying disease, had a longer admission time, and required support in the Paediatric Intensive Care Unit more often than that of the other sub-groups. Just under half (44.7%) of the CCC were carriers of a technological device. Of the total of long stays (>1 month), 71.3% had CCC. CONCLUSIONS: Patients with CCC require long hospital stays, a greater need of intensive care, and use of technology. New approaches to treatment and follow-up need to be established. They should be cost-effective, and at the same time decrease the impact of the disease on the children and their family.
RESUMEN
Human metapneumovirus was discovered recently respiratory virus implicated in both upper and lower respiratory tract infection. In children, the clinical symptoms of human metapneumovirus are similar to those produced by respiratory syncytial virus, ranging from mild to severe diseases such as bronchiolitis and pneumonia. The aim of the present study was to describe the prevalence of human metapneumovirus and other common respiratory viruses among admitted to hospital infants. From January 2006 to June 2006, 99 nasopharyngeal aspirates were collected from hospitalized children younger than 12 months in order to study respiratory viruses. Human metapneumovirus detection was performed by cell culture and two RT-PCR targeting on polymerase and fusion genes. The latter gene was used for phylogenetic analysis. In 67/99 children (67%) at least one viral pathogen was identified, the viruses detected most frequently were respiratory syncytial virus (35%), human metapneumovirus (25%) and rhinovirus (19%). The results obtained in this study, show that: (1) human metapneumovirus is one of the most important viruses among children less than 12 months; (2) children infected with human metapneumovirus were significantly older than those infected by respiratory syncytial virus; (3) human metapneumovirus was associated more frequently with pneumonia whereas respiratory syncytial virus was only detected in patients with bronchiolitis; (4) there was a clear epidemiological succession pattern with only a small overlap among the viruses detected most frequently; (5) all human metapneumovirus samples were clustered within sublineage A2.
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Hospitalización , Metapneumovirus/aislamiento & purificación , Infecciones por Paramyxoviridae/epidemiología , Infecciones del Sistema Respiratorio/epidemiología , Animales , Línea Celular , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Metapneumovirus/clasificación , Metapneumovirus/genética , Infecciones por Paramyxoviridae/virología , Prevalencia , Infecciones del Sistema Respiratorio/virología , Reacción en Cadena de la Polimerasa de Transcriptasa Inversa , España/epidemiología , Cultivo de Virus , Virosis/epidemiología , Virosis/virología , Virus/clasificación , Virus/aislamiento & purificaciónRESUMEN
Epstein-Barr virus (EBV) infection results in a spectrum of clinical manifestations. The host immune response to EBV plays a key role in the extent and degree of clinical features, which in children under 4 years of age are usually mild, non-specific and self-limiting. A 2-year-old boy in whom no known immune disorder could be found presented with acute acalculous cholecystitis, renal dysfunction with massive proteinuria, ascites, pleural effusion, minimal peripheral oedema and a severe systemic inflammatory response. Improvement occurred after initiation of corticosteroids and antiviral treatment with gancyclovir. In severely symptomatic or complicated EBV infection, a primary immunodeficiency must be suspected. If a primary immunodeficiency has been ruled out, the correct management of severe EBV infection in the immunocompetent host remains controversial.
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Colecistitis/complicaciones , Colecistitis/etiología , Infecciones por Virus de Epstein-Barr/complicaciones , Infecciones por Virus de Epstein-Barr/diagnóstico , Herpesvirus Humano 4/aislamiento & purificación , Síndrome Nefrótico/complicaciones , Síndrome Nefrótico/etiología , Corticoesteroides/administración & dosificación , Antiinflamatorios/administración & dosificación , Antivirales/administración & dosificación , Preescolar , Colecistitis/tratamiento farmacológico , Colecistitis/patología , Infecciones por Virus de Epstein-Barr/tratamiento farmacológico , Infecciones por Virus de Epstein-Barr/patología , Ganciclovir/administración & dosificación , Humanos , Masculino , Síndrome Nefrótico/tratamiento farmacológico , Síndrome Nefrótico/patología , Resultado del TratamientoRESUMEN
Introducción: En las últimas décadas se ha incrementado el número de pacientes crónicos complejos (PCC). Existen pocos datos referentes a la prevalencia de PCC en población pediátrica y su impacto en los ingresos hospitalarios. Los objetivos principales de este trabajo son determinar en nuestro medio la proporción de PCC en hospitalización pediátrica y compararla con la de otros grupos de pacientes ingresados (agudos y crónicos). Pacientes y métodos: Estudio descriptivo retrospectivo realizado en un hospital materno-infantil terciario (de diciembre de 2016 a noviembre de 2017). Se incluyó a todos los pacientes ingresados en el Servicio de Pediatría con una periodicidad quincenal. Se recogieron una serie de variables demográficas, clínicas y de gestión. Para identificar el grado de complejidad de los pacientes se utilizó el sistema de clasificación Clinical Risk Group (CRG) y se crearon 3grupos: agudos, crónicos y PCC. Para el análisis estadístico se usó SPSS v24. Resultados: Se incluyó a 1.433 pacientes. La proporción de PCC en la planta de Pediatría fue del 14,4%. Los PCC tenían mayor edad, ingresaron principalmente por descompensación o progresión de su enfermedad de base, estuvieron ingresados más tiempo y precisaron soporte en la Unidad de Cuidados Intensivos Pediátricos con mayor frecuencia que los otros subgupos. Un 44,7% de los PCC era portador de dispositivos tecnológicos. Del total de largas estancias (>1 mes), el 71,3% fue de PCC. Conclusiones: Los PCC requieren de estancias hospitalarias largas, tienen mayor necesidad de cuidados intensivos y uso de tecnología. Es necesario plantear nuevos enfoques de tratamiento y seguimiento que sean costo-eficientes y que, a la vez, disminuyan el impacto de la enfermedad en el niño y su familia. (AU)
Introduction: The number of patients with complex chronic conditions (CCC) has increased in the last 20 years or so. There is limited data as regards the prevalence of CCC in the paediatric population and its impact on hospital admissions. The main objectives of this study are to determine the proportion of CCC in the paediatric hospital population and compare them with other groups of patients admitted (acute and chronic). Patients and methods: A descriptive, retrospective study was carried out in a tertiary maternity-paediatric hospital (from December 2016 to November 2017). All patients admitted into the Paediatric Department were recruited with a fortnightly frequency. A series of demographic, clinical, and pregnancy data were collected. In order to identify the level of complexity of the patients, the Clinical Risk Group (CRG) was used, with 3groups being created: acute, chronic, and CCC. Statistics analysis was performed using SPSS v24. Results: A total of 1,433 patients were included. The proportion of CCC on the Paediatric Ward was 14.4%. The CCC were older patients, mainly admitted due to decompensation or progression of their underlying disease, had a longer admission time, and required support in the Paediatric Intensive Care Unit more often than that of the other sub-groups. Just under half (44.7%) of the CCC were carriers of a technological device. Of the total of long stays (>1 month), 71.3% had CCC. Conclusions: Patients with CCC require long hospital stays, a greater need of intensive care, and use of technology. New approaches to treatment and follow-up need to be established. They should be cost-effective, and at the same time decrease the impact of the disease on the children and their family. (AU)
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Humanos , Preescolar , Niño , Enfermedad Crónica , Pediatría , Hospitalización , Servicios de Salud del Niño , 28599 , Epidemiología Descriptiva , Estudios Retrospectivos , EspañaRESUMEN
No disponible
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Humanos , Niño , Enfermedad Crónica/terapia , Atención al Paciente/métodos , Cuidado del NiñoRESUMEN
The frequency of apnea in infants <12 months of age admitted with acute bronchiolitis was 5.16% (95% confidence interval: 3.94-6.72). Most commonly detected viruses in the 51 apneic infants were respiratory syncytial virus (33.3%), rhinovirus (13.7%) and viral coinfections (23.5%). Young age and prematurity were the main risk factors for apnea independent of the respiratory syncytial virus status. Non-respiratory syncytial virus infants had a higher rate of prematurity.
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Apnea/epidemiología , Bronquiolitis Viral/epidemiología , Infecciones por Virus Sincitial Respiratorio/complicaciones , Virus Sincitiales Respiratorios , Infecciones del Sistema Respiratorio/epidemiología , Apnea/virología , Bronquiolitis Viral/virología , Coinfección , Humanos , Lactante , Recién Nacido , Infecciones por Picornaviridae/virología , Nacimiento Prematuro , Estudios Prospectivos , Infecciones del Sistema Respiratorio/virología , Rhinovirus , Factores de RiesgoRESUMEN
Viral load (VL) of human metapneumovirus and human bocavirus in infants <12 months admitted for bronchiolitis was analyzed. VL correlated with length of hospital stay in both viruses, human metapneumovirus VL with the duration oxygen therapy and human bocavirus VL inversely with days of respiratory effort before admission. Infants coinfected by other viruses were younger, but no differences were seen regarding VL.
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Bronquiolitis/virología , Bocavirus Humano/aislamiento & purificación , Metapneumovirus/aislamiento & purificación , Infecciones por Paramyxoviridae/virología , Infecciones por Parvoviridae/virología , Carga Viral , Bronquiolitis/patología , Femenino , Humanos , Lactante , Tiempo de Internación , Masculino , Terapia por Inhalación de Oxígeno , Infecciones por Paramyxoviridae/patología , Infecciones por Parvoviridae/patologíaRESUMEN
BACKGROUND: The role of respiratory viruses in the pathogenesis of bronchiolitis was re-evaluated with the use of molecular methods such as PCR for virus detection. Whether specific viruses or the classical clinical risk factors are more important in determining severe bronchiolitis is not well established. AIM: To analyze the specific viruses and clinical variables that can predict severe bronchiolitis at admission. METHODS: Nasopharyngeal aspirates were prospectively collected from 484 children <12 months admitted to the pediatrics ward or PICU at Universitary Hospital Sant Joan de Déu (Barcelona, Spain) for bronchiolitis from October 2007 to October 2008. Clinical and demographic data were collected. Sixteen respiratory viruses were studied using PCR. Severity was assessed with a bronchiolitis clinical score (BCS). RESULTS: Four hundred ten infants that tested positive for respiratory viruses were analyzed. Mixed viral infections did not increase the severity of the disease. Rhinovirus was associated with severe BCS in univariate analysis (P = 0.041), but in the multivariate logistic regression including viruses and clinical data only bronchopulmonary dysplasia (OR 7.2; 95% CI 1.2-43.3), congenital heart disease (OR 4.7; 95% CI 1.1-19.9), prematurity (OR 2.6; 95% CI 1.3-5.1), and fever (OR 1.8, 95% CI 1.1-3.1) showed statistical significance for predicting severe BCS. CONCLUSIONS: Classical clinical risk factors have more weight in predicting a severe BCS in infants with acute bronchiolitis than the involved viruses.
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Bronquiolitis/epidemiología , Bronquiolitis/virología , Femenino , Humanos , Lactante , Recién Nacido , Modelos Logísticos , Masculino , Análisis Multivariante , Reacción en Cadena de la Polimerasa , Factores de Riesgo , Índice de Severidad de la EnfermedadAsunto(s)
Humanos , Masculino , Preescolar , Insuficiencia Cardíaca/complicaciones , Insuficiencia Cardíaca/diagnóstico , Electrocardiografía/instrumentación , Electrocardiografía/métodos , Electrocardiografía , Taponamiento Cardíaco/complicaciones , Taponamiento Cardíaco/diagnóstico , Pericardiocentesis/instrumentación , Pericardiocentesis/métodos , Insuficiencia Cardíaca/fisiopatología , Insuficiencia Cardíaca , Frecuencia Respiratoria/fisiología , Tomografía Computarizada de Emisión , Radiografía Torácica/métodos , Radiografía Torácica , Taponamiento Cardíaco , PericardiocentesisRESUMEN
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No disponible
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Humanos , Masculino , Femenino , Niño , Bronquiolitis , Radiografía Torácica , Fiebre/etiología , Antibacterianos/uso terapéutico , Bronquiolitis/tratamiento farmacológico , Índice de Severidad de la EnfermedadRESUMEN
No disponible